Swallowing Difficulties in Progressive
Supranuclear Palsy: A Personal Perspective
Bertrand L Jaber, M.D.,
Division of Nephrology,
Department of Medicine,
New England Medical Center Hospital, Boston, MA
Adapted from an article in the PSP Advocate - Fourth Quarter,
1998
My father was diagnosed with PSP in 1995 at the age of 70. He
had been suffering from recurrent falls and visual difficulties
for four years. At the time of diagnosis, a modified barium
swallow study confirmed aspiration of clear liquids into his
trachea, putting him at risk for pneumonia. At that time, the
dietician recommended that all food and drinks have the
consistency of thick-set yogurt. Soon afterward, I painfully
discussed with my father the need to consider a feeding tube at
some point. He deferred this decision to a later date. Over the
following three years, the disease progressed and he became
increasingly dependent on my mother who cared for him at home. It
is a testament to my mother's determination and devotion that
my father's health and weight were maintained for such a long
period of time. Early this year, I urgently flew back home to
find out that he had lost significant weight and feeding him by
mouth had become an unbearable and inhumane ritual that neither
of my parents could withstand. I felt compelled to revisit the
need for a feeding tube, after having conferred with my mother
and my two brothers and having obtained their support. I sadly
succeeded in convincing him. The feeding tube was placed by a
gastroenterologist under local anesthesia without any medical
complications. However, this intervention was a major turn in my
father's helpless resignation to this new physical handicap.
In the next six months, his physical condition continued to
deteriorate. He stopped ambulating and became bed-ridden,
requiring total care. He continued to lose weight despite tube
feeding. In fact, the feeding formula was often reassessed due to
excessive diarrhea or constipation. He died at home on June 29,
1998 following an episode of aspiration.
Facing the dilemma
Few people are equipped to deal with the painful ethical
decisions that arise when a patient with PSP reaches an advanced
stage of the illness My experience both as a physician and as the
son of a patient with PSP instigated me to review the issue of
tube feeding. Whereas published literature on issues of tube
feeding in elderly patients with dementia is quite elaborate,
little is known of the role, timing and efficacy of tube feeding
in PSP. The relationship between nutrition, aging and disease is
quite complex. Indeed, the normal age-related decline in the host
defense systems coupled to nutritional deprivation, may
contribute to the increased susceptibility of the elderly
individual to infection.
Whereas most physicians would agree that patients with PSP who
have swallowing difficulties (or dysphagia) eventually require a
feeding tube through which semi-liquid food has to be
administered, few are clear on the timing, benefits and risks
associated with this form of feeding, particularly in patients
with advanced disease.
A priority one could argue that tube feeding should be
reserved for patients with advanced stages of PSP, in whom there
is a faiiure to insure minimum nutritional requirements due to
severe dysphagia. However, this decision is often delayed due to
ethical issues, in particular, the inability of the healthcare
provider to confirm whether an intervention (such as the
placement of feeding tube) will help or be distressing to a
patient close to death. I propose that the need for a feeding
tube should be discussed with the patient, and its placement
pursued aggressively as soon as the diagnosis of dysphagia has
been confirmed. If the patient is comfortable with this decision,
I believe that early and intensive nutritional therapy can
actually delay complications related to immobility, prevent
infections, in particular, aspiration pneumonia.
Finally, malnutrition is often associated with vitamin
deficiencies which may further weaken the host defense mechanisms
against PSP, and contribute to the progression of this
degenerative disease. Indeed, free radicals are powerful
substances that can damage cells. Recent research suggests that
free radicals are associated with many degenerative conditions
including the normal aging process, neurological disorders (such
as dementia and Parkinson's disease) and cancer. Antioxidants
are substances that inhibit the damaging effect free radicals,
and vitamin E is one such naturally occurring antioxidant. In the
last issue of the PSP Advocate, there was a report on studies
being launched in patients with PSP to examine the effect of free
radicals on mitochondria, a cell component that is responsible
for energy metabolism. The results of these studies may shed
light on the role of free radicals in PSP, and help researchers
design clinical studies that would target their harmful effects
by using antioxidants. Although there is currently no evidence to
support the use of antioxidants in PSP. I propose that vitamin E,
at a daily dose of 400 lU., is a safe and well tolerated dose,
and may have beneficial effects in the treatment of this
disease.
In conclusion, since aging and nutritional deficiencies may
severely compromise an elderly individuals potential to confront
PSP, it remains to be determined whether early and aggressive
nutritional therapy, once acknowledged by a patient in the early
stages of the disease, may delay its progression.
In memory of
Toufic S. Jaber
Swallowing Diffulties in Progressive Supranuclear Palsy: A
Speech Pathologist's Perspective
Laura Purcell Verdun, M.A., CCC
Adapted from an article in the PSP Advocate - Fourth Quarter,
1998
I would like to start off by recognizing and thanking Dr.
Jaber for sharing his personal experience with PSP and swallowing
difficulties. My comments will reflect some of his, and expand
somewhat on the type of problems that occur with swallowing and
what resources are available to us. Additionally, I will
incorporate some of the issues discussed at the Northern
California PSP symposium in early November.
As many of you know, progressive supranuclear palsy (PSP) is a
neurological disorder that predisposes individuals to swallowing
difficulties. Therefore, it is important that aggressive efforts
are made to evaluate and manage these difficulties to minimize
complications such as malnutrition and aspiration pneumonia,
earlier rather than later in the course of the disease.
Dysphagia, or difficulty swallowing, can be life threatening
particularly when it places an individual at risk for aspiration.
Aspiration is when food, material, or saliva go down "the
wrong way" into the airway, trachea, toward the lungs.
The swallowing evaluation usually consists of first a clinical
examination. The majority of patients with PSP are referred to me
by neurologists. I spend my time with patients and their care
partners elucidating their concerns, their experences and
observations related to the potential swallowing problem. I find
that when families maintain a diary of their observations that
this is particularly useful for developing my management
strategies. Generally during the clinical examination I will make
suggestions to promote safer and easier swallowing. The
suggestions are based on my understanding of normal swallowing
physiology, and the changes I have observed in the numerous
individuals with PSP with whom I have had the opportunity to
work.
This clinical examination may be followed by a modified barium
swallowing study, or videofluoroscopic swallowing study. This is
a dynamic radiographic examination of the swallowing passage
during various consistency and volume presentations. Dr. Jaber
notes that his father had this examination, which documented
aspiration. I must admit that I do not standardly conduct this
examination on all patients with PSP, because I do not believe
that all patients need this examination. Clearly there are those
individuals who are having such great difficulty with swallowing,
that this examination would not provide us with any useful
additional information. However, the decision to proceed with
this examination is made on a case by case basis.
Dr. Jaber reported that they used a very common intervention,
that is to modify the consistency of the foods and liquids his
father was consuming. Thickening liquids will slow the transport
through the mouth and throat. This is important if swallowing is
not well coordinated, weak, or there is a disruption in timing
events. So a patient may have trouble swallowing grape juice, but
making it a little thicker to a nectar consistency may be
manageable. Soft and moist foods are also a common
recommendation, avoiding dry and particulate foods such as nuts,
and highly textured foods such as meats. These are generally
easier to manage in the mouth, and may require less chewing.
Another common suggestion is to take medications with a puree or
pudding consistency, instead of with water or other liquids.
Clearly aspiration in and of itself can place an individual at
risk for developing aspiration pneumonia. However, this is not
likely exclusive. Over time, studies have shown that there are
other contributing factors which can increase an individual's
risk for developing aspiration pneumonia, for example, poor oral
hygiene, bed dependence, and dependence for feeding. Additionally
the presence of a feeding tube does not eliminate the potential
for aspiration pneumonia, and it may potentially increase the
risk. Clearly the development of aspiration pneumonia is a
complicated, multifactorial process.
Dr. Jaber proceeds to share with us his personal decision
making process regarding placement of a feeding tube for his
father. This is a particularly difficult situation, and one that
faces each family dealing with PSP. There is no right or wrong
answer on how to make this decision, or what decision to make.
However, I do believe that it is important policy for medical
professionals and families to approach this topic sooner than
later, so that the patient has the opportunity to communicate
their interests. To some extent I believe this has the potential
minimize the stress of the family making the decision fot their
member with PSP. However, this does not necessarily mean that the
tube should be placed sooner than later. Dr. Jaber addresses this
issue perfectly. There is no research that would guide us on how
to proceed. It is imoortant to understand the risks for
proceeding without a feeding tube, and alternatively, the risks
and responsibilities associated with placement of a feeding tube.
It is possible that early placement of a feeding tube may
minimize the complications Of malnutrition and dehydration, but
this has yet to be proven, particularly in the PSP
population.
I concur wholeheartedly with Dr. Jaber's conclusions. It
is yet undetermined as to whether early and aggressive
nutritional therapy may delay the progression of the disease.
Perhaps it would allow that individual greater medical stability
in with which to cope and minimize complications. My personal
professional goal is that with a more prompt accurate diagnosis
of PSP, patients will be referred on for swallowing intervention
sooner than later, when perhaps little can be offered. I am very
interested in determining if early intervention to address the
anticipated feeding and swallowing difficulties associated with
PSP, while an individual is still tolerating an oral diet,
however with minimal or no evidence of impairment, could prolong
safe, easy, and enjoyable mealtimes. Finally, I suggest that you
refer to the new SPSP brochure on swallowing difficulties in PSP
for additional information.
Laura Purcell
Verdun is a Part Time Assistant Professor at Johns Hopkins
University Department of Otolarynglory. She also works in the
Voice and Speech Section, NINCD, National Institutes of Health.
Laura is a member of the SPSP Board of Directors and has been
active with PSP for over 6 years.
The PSP Advocate is
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